Diagnostic and Interventional Radiology
Head and Neck Imaging - Case Report

CT and MRI findings in X-linked progressive deafness

1.

From the Department of Radiology, Ege University School of Medicine, İzmir, Turkey

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Departments of Radiology, Ege University School of Medicine, İzmir, Turkey

3.

Departments of Otolaryngology, Ege University School of Medicine, İzmir, Turkey

Diagn Interv Radiol 2008; 14: 117-119
Read: 407 Downloads: 305 Published: 03 September 2019

Abstract

Congenital X-linked mixed deafness is a rare anomaly that has typical features and can be diagnosed on the basis of progressive mixed hearing loss and the typical imaging findings. Recognition of these findings may alter the course of treatment and perilymph gushing can be avoided. A 10-year-old male patient presented with a history of progressive hearing loss. Computed tomography of the temporal bones showed bulbous dilatation of the fundi of the internal auditory canals (IAC) and the absence of the bony plates separating the basal turn of the cochleas and IAC. Magnetic resonance imaging demonstrated obliteration of the labyrinthine spaces at the right side.

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